A type II mutation (Glu117stop), induction of allele-specific mRNA degradation and factor XI deficiency

Haematologica. 2005 Dec;90(12):1716-8.

Abstract

The Glu117stop mutation in the factor XI (FXI) gene is the most common cause of FXI deficiency and might cause the disease either by poor secretion/stability of the truncated protein or by decreased mRNA levels. Platelet- and lymphocyte-derived mRNA from three Glu117stop heterozygotes were analyzed by reverse-transcriptase polymerase chain reaction and sequencing, demonstrating allele-specific reduction of FXI Glu117stop mRNA.

Publication types

  • Letter
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Alleles
  • Blood Platelets / metabolism
  • Child
  • Codon, Nonsense*
  • DNA Mutational Analysis
  • Exons / genetics
  • Factor XI / genetics*
  • Factor XI Deficiency / genetics*
  • Heterozygote
  • Humans
  • Lymphocytes / metabolism
  • Male
  • RNA, Messenger / metabolism*
  • Reverse Transcriptase Polymerase Chain Reaction
  • Sequence Analysis, DNA

Substances

  • Codon, Nonsense
  • RNA, Messenger
  • Factor XI